Nonendemic pemphigus foliaceus in children - 01/09/11
Abstract |
Background: Pemphigus foliaceus is a cutaneous, autoimmune, blistering disease comprising two major categories: endemic and sporadic. The endemic form, also known as fogo selvagem, primarily affects children and young adults in rural Brazil. In contrast, the sporadic form of pemphigus foliaceus is generally a disease of the middle-aged and elderly. Objective and methods: Because the sporadic form of pemphigus foliaceus rarely affects children, information specific to this unique group is lacking. We describe a 3-year-old boy with the disease and retrospectively review data from 28 past cases. Results: In comparison to pediatric cases of pemphigus vulgaris, sporadic pemphigus foliaceus in children tends to follow a generally benign course of relatively short duration. However, long-term outcome studies are lacking. A pattern of skin lesions described as “arcuate,” “circinate,” or “polycyclic” appears to be a unique and specific presentation of this disease in children. Occasionally, as in our case, the diagnosis may prove difficult to establish by using routine histology or immunopathology. Conclusion: The commercial availability of antigen-specific techniques such as enzyme-linked immunosorbent assay for serum desmoglein 1 autoantibody should eliminate delay in diagnosis. Hydroxychloroquine may be another treatment option for those children with photodistributed lesions. Further experience and long-term outcome studies in children are needed to determine whether some medication side effects may outweigh the risks from the disease itself. (J Am Acad Dermatol 2002;46:419-22.)
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Funding sources: None. |
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Conflict of interest: None. |
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Reprint requests: Denise W. Metry, MD, Department of Dermatology & Pediatrics, Texas Children's Hospital, Baylor College of Medicine, 6621 Fannin St, MC 3-3315, Houston, TX 77030-2399. E-mail: dwmetry@texaschildrenshospital.org. |
Vol 46 - N° 3
P. 419-422 - mars 2002 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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